Ann Thorac Surg 1997;64:552-554
© 1997 The Society of Thoracic Surgeons
Case Reports
Surgical Management of Intrapericardial Teratoma Diagnosed in Utero
Patrick T. Paw, MD,
Stuart W. Jamieson, FRCS
Division of Cardiothoracic Surgery, University of California, San Diego, San Diego, California
Accepted for publication March 26, 1997.
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Abstract
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Intrapericardial teratoma is a rare, often benign congenital tumor, which frequently leads to respiratory distress and pericardial tamponade shortly after birth. Surgical excision is not only curative but potentially lifesaving, because these lesions often become fatal if not promptly excised. We present a case of one of two twins diagnosed with a large intrapericardial teratoma. The management of this case was complicated by the necessity to consider the well-being of the other (normal) twin in planning the timing of delivery and operation. After the twins were delivered by cesarean section in one operating room, the mass was excised from the infant in an adjoining operating room with cardiopulmonary bypass standby.
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Introduction
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The infant is one of two twins born to a 27-year-old gravida 4, now para 3, Ab 2 woman with an initial screening ultrasound demonstrating normal twin pregnancy. However, subsequent repeat ultrasound at 24 weeks revealed a new pericardial effusion and a large echogenic soft tissue mass in one of the two twins. By 30 weeks of gestational age, this large anteriorly located mass measured 4.5 x 4.0 x 3.5 cm (Fig 1
). Multiple pericardiocenteses were performed due to the development of right atrial diastolic collapse consistent with pericardial tamponade. Increasing ascites was suggestive of hydrops. A cesarian section was planned at the end of the 34 weeks after confirmation of a mature lung profile as evident by a lecithin to sphingomyelin ratio of 2.4. This was thought to be optimal because of the primary concern for the safety of the mother and the normal twin, and also to minimize the risk of the mass causing compression on the heart resulting in pericardial tamponade at the time of vaginal delivery.
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Fig 1. . Ultrasound demonstrating intrapericardial teratoma at a gestational age of 30 weeks. ( LA = left atrium; LV = left ventricle; PCE = pericardial effusion; RV = right ventricle.)
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The concern for the affected twin centered about cardiorespiratory function in the presence of the large tumor. After delivery of this 2,335-g infant, an endotracheal tube was inserted because of respiratory distress and for airway protection. An echocardiogram confirmed the correct twin was being submitted for the operation in an adjoining operating room. Chest radiograph revealed a large chest mass (Fig 2). After a midline sternotomy incision, the pericardium was incised and 40 mL of serosanguinous fluid was obtained. A large intrapericardial mass measuring 9.0 x 6.0 x 3.0 cm was seen on the anterior surface of the heart adherent to the epicardium of both ventricles and right atrium with a broad pedicle attached to the ascending aorta. It was removed entirely. Cardiopulmonary bypass was not required due to the lack of myocardial invasion. The infant was weaned off the ventilator on postoperative day 3 and subsequently discharged home with his parents. The other twin appeared to be normal.
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Fig 2. . (A) Chest radiograph at birth before resection of the mass, demonstrating a large intrapericardial teratoma. (B) Chest radiograph performed after resection of the mass.
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Pathologic examination of the specimen confirmed a well-encapsulated, pink-whitish mass measuring 9.0 x 5.8 x 2.5 cm. It contained multiple cystlike structures consistent with an intrapericardial teratoma (Fig 3).
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Comment
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Of the 56 cases of intrapericardial teratoma reported thus far, only 8 cases have been diagnosed in utero, and 5 associated with hydrops fetalis [1, 2]. This case is unusual in that the patient was one of twins and required careful management to balance the requirements of the other twin. Serial in-utero ultrasounds have demonstrated that tumor growth occurs rapidly between 20 and 40 gestational weeks when these abnormal groups of cells undergo rapid growth and differentiation [3, 4].
Malignant cases of intrapericardial teratoma are uncommon, accounting for only 15% of all such tumors [5]. Typically, malignant teratomas contain no discrete vascular attachment to the heart or great vessels. Prognosis is poor; however, no definitive conclusion can be made regarding survival after excision because of the paucity of such case reports. In utero intrapericardial teratomas are frequently diagnosed by ultrasound performed as part of a routine obstetrical screen, or for hydrops fetalis. This reveals a large pericardial effusion with a mass at the level of the ascending aorta. Infants with evidence of pericardial tamponade but whose lung profile demonstrates hypoplastic lungs may benefit from in utero pericardiocentesis [1, 2]. Preferably, delivery should be via cesarean section once a mature lung profile is obtained, because a rapid rise in cardiac compression during vaginal delivery may result in worsening chest compression by the tumor, resulting in tamponade and death [1].
All intrapericardial teratomas eventually become symptomatic [6, 7]. Infants and neonates frequently present with respiratory distress, pericardial effusion, and cardiac tamponade shortly after birth [1, 2, 4]. The lesion is frequently fatal it not promptly resected [3, 7]. Therefore, treatment by early surgical excision is not only curative but lifesaving, because the tumor is usually benign and patients are frequently born or present with acute cardiorespiratory distress. No cases of recurrence after complete surgical excision have been reported [6]. Spontaneous regression of suspected intrapericardial teratomas also has been reported, but this is rare [4].
Most tumors can be excised without further diagnostic studies including computed tomographic scan and angiography, because approximately 90% of the tumors are attached to the aorta or main pulmonary artery via a vascular stalk or pedicle [7]. This was the case with our patient, although it was also adherent to the right ventricle. Complete surgical excision can usually be accomplished for most benign teratomas without cardiopulmonary bypass. Malignant lesions may benefit from excision under cardiopulmonary bypass or subtotal excision followed by postoperative adjuvant chemotherapy and radiation therapy [8].
In conclusion, intrapericardial teratoma is a rare tumor that often causes severe cardiovascular and respiratory distress in newborns and infants. A carefully planned and coordinated cesarean section followed by immediate surgical resection is not only lifesaving but curative, because most are benign. Operation should be performed without delay once an ultrasound confirms the presence of an intrapericardial teratoma with pericardial effusion. In this case efforts had to be made to ensure the safety of the other normal fetus.
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Footnotes
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Address reprint requests to Dr Jamieson, Division of Cardiothoracic Surgery, University of California, San Diego, 200 W Arbor Dr, #8892, San Diego, CA 92103-8892.
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References
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- Dehner LP. Gonadal and extragonadal germ cell neoplasia of childhood. Hum Pathol 1983;14:493–511.[Medline]
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Abstract
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